Top 10 Pulmonary Fibrosis Stories of 2020
During 2020, Pulmonary Fibrosis News held to its mission of providing daily coverage of breakthrough discoveries, promising therapies, clinical trials, and other news related to pulmonary fibrosis (PF). As we look forward to continuing to bring news and relevant information to patients, family members, and caregivers dealing with PF in 2021, here are the top 10 most-read articles of 2020, with a brief description of what made them interesting to the PF community.
As 2020 was marked by the COVID-19 pandemic, the Centers for Medicare and Medicaid Services issued new guidelines and decided to waive the need for in-person testing and a signature at delivery for supplemental oxygen to PF patients. The new guidelines, which received the support of the Pulmonary Fibrosis Foundation and will remain in effect throughout the pandemic, were implemented to lessen the risk of exposing PF patients to the virus that causes COVID-19 while allowing them to continue with oxygen therapy. Prescribed to many PF patients, this therapy helps to raise blood oxygen levels and improve breathing.
A Finnish study reported that following an acute exacerbation, people with idiopathic pulmonary fibrosis (IPF) have shorter survival times than do those with other interstitial lung diseases (ILDs), an umbrella term for more than 200 lung disorders. The study showed that IPF patients lived a median of 2.6 months after being hospitalized for an acute exacerbation, or flare, while those with other ILDs lived a median of 21 months.
A study reported that statins — a class of medications used to lower cholesterol levels and commonly prescribed to reduce the risk of heart disease and stroke — may be an effective treatment for IPF. Using a mouse model of induced PF, researchers showed that simvastatin, a type of statin, reduced collagen accumulation, and fibrosis (scarring) markers and tissue remodeling triggered by fibrosis.
The experimental therapy GED-0507, by Nogra Pharma, may offer a new way of treating PF, according to data from a preclinical study. Results in a mouse model of induced PF showed that preventive treatment with GED-0507 limited body weight loss and significantly reduced lung fibrosis to a similar or greater extent than Ofev (nintedanib, marketed by Boehringer Ingelheim) and Esbriet (pirfenidone, marketed by Genentech, a Roche company), two approved anti-fibrotic therapies.
Our sixth most-read article of 2020 highlighted the release of the documentary “Beyond Breathless,” focusing on the experiences of real people living with rare ILDs, as well as their families, caretakers, and healthcare providers. The documentary, by Boehringer Ingelheim, features the actress and producer Queen Latifah and the former NY Yankee Bernie Williams.
Another experimental therapy, FibroGen’s pamrevlumab, may be more effective than Esbriet and Ofev at slowing disease progression in people with IPF. A pooled meta-analysis of eight clinical trials showed that while all three therapies significantly slowed lung function decline in IPF patients relative to a placebo, pamrevlumab was associated with a less marked worsening. Its researchers cautioned, however, that this work lacked a head-to-head comparison of these different medications.
The antibiotic azithromycin (AZT), commonly used to fight bacterial infections, was found to have anti-fibrotic effects in cells from people with IPF, reducing the levels of such pro-fibrotic markers as the Col1A1 protein, fibronectin, and the protein alpha smooth-muscle actin (alpha-SMA). Moreover, IPF fibroblasts — cells promoting fibrosis — were found to be more sensitive to AZT treatment than healthy fibroblasts, with the treatment triggering their death.
Our third most-read article of 2020 reported on the Pulmonary Fibrosis Foundation’s release explaining how fibrosis in the lungs caused by COVID-19 is distinct from what generally occurs in people with an ILD.
COVID-19, a viral infection that affects the airways and the lungs, can cause pneumonia that progresses to acute respiratory distress syndrome (ARDS) in certain people. ARDS is characterized by the buildup of fluid in the lungs, preventing oxygen from reaching the bloodstream. Patients who develop ARDS almost always require a ventilator to survive, and some later on develop lung fibrosis. The scarring that follows ARDS, however, typically does not progress over time as is seen in people with ILDs, although it too can be severe and limiting.
A delayed IPF diagnosis, one coming more than a year after the onset of symptoms, is linked with a higher frequency of conditions affecting the heart, stomach, and esophagus, according to an analysis of patient records from the Idiopathic Pulmonary Fibrosis Prospective Outcomes (IPF-PRO) Registry. Data revealed that patients waiting longer for a correct diagnosis were more likely to experience gastro-esophageal reflux disease, obstructive sleep apnea, coronary artery disease, and cardiac abnormalities, including atrial fibrillation, atrial flutter, deep vein thrombosis, and pulmonary embolism. The risk of death or need for a lung transplant did not appear to be influenced by the length of time to diagnosis, the study noted.
The most-read PF article of 2020 reported how oral dietary supplements of an antioxidant found in green tea — called epigallocatechin-3-gallate (EGCG) — may halt and even reverse the lungs’ pro-fibrotic state in people with IPF, according to a small U.S. study.
EGCG blocks the activity of key proteins involved in lung fibrosis, including fibronectin, type 1 collagen, and alpha-SMA. Daily treatment with EGCG for 14 days led to a normalization of the levels of all these key proteins, suggestive of a reversion of the lung’s pro-fibrotic state. Treatment also led to a significant drop in the levels of two blood biomarkers of IPF progression, cartilage oligomeric matrix protein and periostin.
At Pulmonary Fibrosis News, we hope that these articles, along our reporting throughout 2021, help inform and improve the lives of everyone dealing with PF.
We wish all our readers a happy and bright 2021.